Acta Neuropathologica

, Volume 98, Issue 1, pp 48–54

Colonic enteric nervous system in patients with familial amyloidotic neuropathy

  • Intissar Anan
  • Magdy El-Salhy
  • Yukio Ando
  • Sture Forsgren
  • Nils Nyhlin
  • Hisayasu Terazaki
  • Naomi Sakashita
  • O. B. Suhr
Regular paper

DOI: 10.1007/s004010051050

Cite this article as:
Anan, I., El-Salhy, M., Ando, Y. et al. Acta Neuropathol (1999) 98: 48. doi:10.1007/s004010051050

Abstract

The colonic enteric nervous system was investigated in autopsy specimens from 12 patients with familial amyloidotic neuropathy (FAP) and 9 controls. The infiltration of amyloid deposits in the enteric nervous system was studied by double staining for amyloid and nerve elements. The myenteric plexus was immunostained for protein gene product (PGP) 9.5, vasoactive intestinal peptide (VIP), substance P and nitric oxide synthase (NOS). The immunostained nerve elements were quantified by computerised image analysis. Double staining revealed that there was no amyloid infiltration in the ganglia, or in the nerve fibres in the colonic enteric nervous system of FAP patients. The relative volume density of PGP 9.5-immunoreactive nerve fibres in both the circular and the longitudinal muscle layers in FAP patients did not differ significantly from that of controls. The relative volume density of VIP-immunoreactive nerve fibres in the circular muscle layer was significantly decreased in FAP patients compared with controls, but not in the longitudinal layer. The number of VIP-immunoreactive neurons/mm2 myenteric ganglia was significantly decreased in FAP patients. There were no statistical differences in the relative volume density for substance P- and NOS-immunoreactive nerve fibres between FAP patients and controls, nor was there any difference between FAP patients and controls regarding the number of NOS- and substance P-immunoreactive neurons/mm2 myenteric ganglia. It is concluded that the colonic enteric nervous system as a whole is intact and is not damaged by amyloid infiltration. The present observation of a reduction of VIP-immunoreactive nerve fibres and neurons in myenteric plexus of FAP patients might be one of the factors that contribute to the motility disorders seen in FAP patients.

Key words Familial amyloidotic polyneuropathyProtein gene product 9.5Vasoactive intestinalpolypeptideNitric oxide synthaseSubstance PAmyloid

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • Intissar Anan
    • 1
  • Magdy El-Salhy
    • 1
  • Yukio Ando
    • 1
  • Sture Forsgren
    • 2
  • Nils Nyhlin
    • 1
  • Hisayasu Terazaki
    • 3
  • Naomi Sakashita
    • 4
  • O. B. Suhr
    • 1
  1. 1.Department of Medicine, Gastroenterology Section, Umeå University Hospital and University, S-901 85 Umeå, Sweden e-mail: ole.suhr@medicin.umu.se Tel.: +46-90-7851383, Fax: +46-90-143986SE
  2. 2.Department of Anatomy, Umeå University Hospital and University, Umeå, SwedenSE
  3. 3.First Department of Internal Medicine, Kumamoto University School of Medicine, Kumamoto, JapanJP
  4. 4.Second Department of Pathology, Kumamoto University School of Medicine, Kumamoto, JapanJP